Familial florid cemento-osseous dysplasia--a case report and review of the literature.
Cemento-osseous dysplasia is a fairly common lesion of the jaws originating from the elements of the periodontal ligament. Most lesions do not have a hereditary basis, however a few familial cases have been documented. This is the first report of the florid form of cemento-osseous dysplasia in an African family. The mother, son and one daughter exhibited multiple sclerotic cemental masses of the jaws with large tumour-like swellings in the symphyseal area of the mandible. In the case of the mother the lesions had become infected. The familial form of cemento-osseous dysplasia differs from the non-hereditary type in that the lesions present in children, all cases are florid in nature, there is no predilection for black females, the rate of growth appears to be more rapid and the symphyseal area of the mandible is most severely affected. The mode of transmission appears to be autosomal dominant with variable phenotypic expression.
Coleman
H
H
Department of Oral Pathology, University of the Witwatersrand, Johannesburg, Wits.
Altini
M
M
Kieser
J
J
Nissenbaum
M
M
D000293:Adolescent; D000328:Adult; D044383:Black People; D002485:Cementoma / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D002648:Child; D003739:Dental Cementum / Q000473:pathology / Q000601:surgery; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007571:Jaw Diseases / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D007573:Jaw Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D008297:Male; D008334:Mandible / Q000473:pathology / Q000601:surgery; D010375:Pedigree; D012086:Reoperation